Hepatosplenic T-cell lymphoma of αβ lineage in a 16-year-old boy presenting with hemolytic anemia and thrombocytopenia

The authors report an unusual case of peripheral T-cell lymphoma in a 16-year-old boy who presented initially with jaundice, splenomegaly, anemia, and thrombocytopenia. A lymphoma was found subsequently in the spleen, which was infiltrated extensively in the red pulp by medium-sized, blastic-appearing lymphoma cells. Immunologic characterization of these cells revealed positivity for CD3, CD5, CD45RO, CD56, and T-cell intracellular antigen (TIA), and negativity for CD2, CD3, CD4, CD8, CD57, CD34, and terminal deoxynucleotidyl transferase (TdT). Conventional cytogenetic studies revealed the presence of isochromosome 7q. On follow up, this patient deteriorated rapidly, with evidence of liver and bone marrow involvement. Although the overall clinical and pathologic features of this disease were characteristic of hepatosplenic gamma delta T-cell lymphoma, the T-cell receptor of this tumor showed an immunophenotype of alpha beta not gamma delta lineage. Using the Southern blot technique, the authors demonstrated monoclonal gene rearrangement of the T-cell receptor beta-chain. Thus, they confirmed the existence of hepatosplenic alpha beta T-cell lymphoma. In view of its overall similarity to hepatosplenic gamma delta T-cell lymphoma, this unusual entity probably represents a slight biologic variation of the same disease.